Isomerism of the right atrial appendages with bilateral eparterial bronchi: clinical challenges and surgical management guided by 3D cardiac computed tomography

Isomerism of the right atrial appendages is a rare congenital disorder marked by abnormal lateralization of the thoracoabdominal organs, often associated with complex cardiac malformations and asplenia. We report a neonate with discordant ventriculo-arterial connections, an anterior and right-sided aorta, total anomalous pulmonary venous return, and bilateral eparterial bronchi, identified using cardiac computed tomography with three-dimensional (3D) reconstruction. The patient underwent staged surgical interventions including a Blalock-Taussig shunt and Glenn procedure. Postoperative complications included gastroesophageal reflux and respiratory distress requiring multidisciplinary care. While not intended to guide generalized management, this case illustrates the utility of multimodality imaging and surgical planning in addressing the complex anatomy associated with isomerism of the right atrial appendages.